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PubMed1990

Reticulohistiocytoma cutis--review of 15 cases and an association with systemic vasculitis in two cases.

We reviewed 15 cases of reticulohistiocytoma cutis (RHC). Three cases were of solitary lesions. Four cases were associated with a destructive arthritis but no other systemic features. One additional patient had a destructive arthritis and lesions of reticulohistiocytoma in synovium, larynx, and mucosa. Three patients had associated xanthelasma. Two cases were associated with internal malignancy (metastatic malignant melanoma, adenocarcinoma of the bowel). Two cases were sequentially associated with systemic vasculitis (Wegener's granulomatosis, periarteritis nodosa). This occurrence of systemic vasculitis in RHC patients is unique. RHC may have variable cutaneous and systemic manifestations and associations with malignancy and immunoinflammatory diseases, including vasculitis.

Adult↗
PubMedDate not supplied

Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature.

Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer from interstitial keratitis and sudden onset of tinnitus and hearing loss. Few cases (around 150) have been published and thus it is difficult to determine the percentage of patients with underlying systemic disorders such as systemic vasculitis. The variety of systemic manifestations is large and includes fever, splenomegaly, lymphadenopath, and musculoskeletal complaints. Systemic vasculitis can be seen in around 10% of cases and may involve the large vessels, appearing as Takayasu-like vasculitis with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While corticosteroids have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf patients has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two patients with a wide clinical spectrum of symptoms from local disease restricted to the eyes and ears to a widespread vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.

Adolescent↗
PubMed1993

Identification of haptoglobin as an angiogenic factor in sera from patients with systemic vasculitis.

Angiogenesis is an important process in chronic inflammatory diseases. We observed that sera from patients with systemic vasculitis stimulated angiogenesis in an in vitro model using human umbilical vein endothelial cells cultured on a basement membrane (Matrigel) substrate. After 40% ammonium sulfate precipitation, angiogenic activity remained in the low molecular weight fraction and could be inactivated by heat. SDS-page of serum FPLC fractions exhibiting maximal angiogenic activity demonstrated two prominent species of 45 and 16-20 kD in patients' sera. These bands were much less apparent in sera obtained from control subjects. Amino-terminal sequencing of the 45-kD protein demonstrated that it was haptoglobin. Purified haptoglobin stimulated angiogenesis in a dose-dependent manner. The angiogenic activity of vasculitis patients' sera was partially inhibited by an antihaptoglobin antibody. Furthermore, serum haptoglobin levels in vasculitis patients correlated both with disease and angiogenic activity. Haptoglobin angiogenic activity was confirmed in two in vivo models using an implanted disc and a subcutaneous injection of basement membrane. Stimulation of angiogenesis is a newly recognized biological function of haptoglobin. The increased levels of haptoglobin found in chronic inflammatory conditions may play an important role in tissue repair. In systemic vasculitis, haptoglobin might also compensate for ischemia by promoting development of collateral vessels.

Amino Acid Sequence↗
PubMed1997

Methotrexate use in systemic vasculitis.

Although GS and CYC have been important agents in improving the outcome and survival of patients with systemic vasculitis, they carry their own risk of drug-induced morbidity and mortality. It has also become apparent that these medications are not the final answer in disease management because some forms of vasculitis have the potential to relapse or be treatment resistant. For these reasons, the pursuit of effective, less toxic therapeutic alternatives is critical. Initial results from the use of MTX in systemic vasculitis have been encouraging. Although drug-related toxicity and disease relapse have still been found to occur, MTX appears to be a valuable addition in the treatment of vasculitis. Further studies will be necessary to determine the optimal way that this agent may be used to safely and effectively manage vasculitic disease.

Antirheumatic Agents↗
PubMed1995

Distribution of MHC class II alleles in primary systemic vasculitis.

Previous studies have shown a number of different associations between major histocompatibility complex (MHC) alleles and primary systemic vasculitis. Disease heterogeneity and the lack of specificity of certain MHC typing techniques may have contributed to the lack of consistency in those studies. We therefore studied a relatively homogeneous group of 94 patients with Wegener's granulomatosis, microscopic polyangiitis, or renal-limited vasculitis using molecular techniques that allow more precise assignment of MHC genotype. DNA was prepared from peripheral blood and DRB1 genotype determined by Taq restriction fragment length polymorphism. DQB1 and DPB1 genotype were assigned by polymerase chain reaction amplification followed by probing with allele-specific oligonucleotides. Specificity of associated anti-neutrophil cytoplasm antibodies (ANCA) was determined where possible by solid phase immunoassays using purified proteinase 3 (PR3) and myeloperoxidase (MPO). After correction for multiple comparisons there were no significant differences in the distribution of DRB1, DQB1 and DPB1 alleles between a local control group (N = 90 for DRB1, N = 50 for DQB1 and DPB1) and the patient group as a whole (N = 94) or two a priori defined subgroups (anti-PR3 positive, N = 35; anti-MPO positive, N = 22). We have therefore found no significant association between primary systemic vasculitis and any MHC class II allele. This, together with the fact that previous smaller studies have shown no consistent association, suggests that any such association is very weak, if it exists at all.

Alleles↗
PubMed2006

EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis.

OBJECTIVES: To develop the European League Against Rheumatism (EULAR) recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis. METHODS: An expert consensus group was formed consisting of rheumatologists, nephrologists and specialists in internal medicine representing five European countries and the USA, a clinical epidemiologist and representatives from regulatory agencies. Using an evidence-based and expert opinion-based approach in accordance with the standardised EULAR operating procedures, the group identified nine topics for a systematic literature search through a modified Delphi technique. On the basis of research questions posed by the group, recommendations were derived for conducting clinical studies and/or clinical trials in systemic vasculitis. RESULTS: Based on the results of the literature research, the expert committee concluded that sufficient evidence to formulate guidelines on conducting clinical trials was available only for anti-neutrophil cytoplasm antibody-associated vasculitides (AAV). It was therefore decided to focus the recommendations on these diseases. Recommendations for conducting clinical trials in AAV were elaborated and are presented in this summary document. It was decided to consider vasculitis-specific issues rather than general issues of trial methodology. The recommendations deal with the following areas related to clinical studies of vasculitis: definitions of disease, activity states, outcome measures, eligibility criteria, trial design including relevant end points, and biomarkers. A number of aspects of trial methodology were deemed important for future research. CONCLUSIONS: On the basis of expert opinion, recommendations for conducting clinical trials in AAV were formulated. Furthermore, the expert committee identified a strong need for well-designed research in non-AAV systemic vasculitides.

Antibodies↗
PubMed1996

The renal histopathology in systemic vasculitis: an international survey study of inter- and intra-observer agreement.

In order to relate histopathological findings of the kidney in systemic vasculitis to renal outcome, scoring of various morphological parameters is necessary. Therefore, we conducted a standardization study for evaluating renal biopsies from patients with systemic vasculitis. Four experienced renal pathologists from four European centres joined in the study. A scoring protocol was devised that required the observers to score an extensive number of histopathological lesions either quantitatively (as a percentage of the total number of glomeruli) or dichotomously (on a present/absent scale). Twenty renal biopsies were scored individually by all the observers, from which the inter-observer variability was analysed. Ten randomly chosen biopsies were scored again, in order to obtain the intra-observer variability. For inter-observer agreement, the evaluation of the quantitative variables was satisfactory for both rounds (0.55 < or = Kendall's W < or = 0.95 and 0.59 < or = W < or = 0.96, respectively, with all P < 0.05). However, the inter-observer agreement for the dichotomous data was poor (kappa < or = 0.30 in more than half of the parameters in both rounds). Also the data on intra-observer agreement showed more favourable results for the analysis of the quantitative data (Pearson's r > 0.45 in more than 85% of the variables in both rounds) than for the dichotomous scoring system (kappa < or = 0.30 in more than half of the variables). It is concluded that even between experienced renal pathologists discrepancies occur in scoring kidney biopsies. Inter- and intra-observer agreement is greater if a quantitative method for reviewing the biopsies is applied that requires the observers to score the tissue specimens systematically.

Biopsy↗
PubMed1999

The activation of the neutrophil respiratory burst by anti-neutrophil cytoplasm autoantibody (ANCA) from patients with systemic vasculitis requires tyrosine kinases and protein kinase C activation.

The ability of antineutrophil cytoplasm autoantibodies (ANCA) from patients with systemic vasculitis to stimulate protein kinase C (PKC) and tyrosine kinases was examined in human neutrophils. Using the superoxide dismutase-inhibitable reduction of ferricytochrome C, the kinetics of ANCA-induced superoxide (O2-) production were characterized and subsequently manipulated by specific inhibitors of PKC and tyrosine kinases. With this approach, ANCA IgG, but not normal IgG or ANCA F(ab')2 fragments caused a time and dose dependent release of O2- from TNF-alpha primed neutrophils. The kinetics of ANCA-induced O2- production showed an initial 10-15 min lag phase compared to the N-formyl-L-methionyl-L-leucyl-L-phenylalanine response, suggesting differences in the signalling pathways recruited by these two stimuli. Inhibitor studies revealed that ANCA-activation involved members of both the Ca2+-dependent and -independent PKC isoforms and also tyrosine kinases. ANCA IgG resulted in the translocation of the betaII isoform of PKC at a time corresponding to the end of the lag phase of O2- production, suggesting that PKC activity may be instrumental in processes regulating the activity of the NADPH oxidase in response to ANCA. Tyrosine phosphorylation of numerous proteins also peaked 10-15 min after stimulation with ANCA but not normal IgG. These data suggest that PKC and tyrosine kinases regulate O2- production from neutrophils stimulated with autoantibodies from patients with systemic vasculitis.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1993

Clinical significance of anti-endothelial cell antibodies in systemic vasculitis: a longitudinal study comparing anti-endothelial cell antibodies and anti-neutrophil cytoplasm antibodies.

Ten patients with systemic vasculitis, all positive for anti-neutrophil cytoplasm antibody (ANCA), were followed for a mean of 36 weeks to determine the prevalence of anti-endothelial cell antibodies (AECAs) and the relationship between AECAs, ANCAs, and disease activity. Anti-endothelial cell antibodies were detected in eight (80%) patients at some time during the study. The levels of both AECAs and ANCAs changed with time, and these autoantibodies were present in 48% and 63% of the total 100 serum samples, respectively. Eighteen clinical remissions were observed; in 16 (88.9%) cases the level of ANCAs dropped. Fifteen (83.3%) of the 18 remissions were among the AECA-positive patients and the level of AECAs decreased in 13 (86.7%) instances (P = not significant). There were 11 episodes of disease relapse; all were associated with an increase in the level of ANCAs. Nine (81.8%) of the 11 relapses were among AECA-positive patients, and the level of AECAs increased in eight (88.9%) cases (P = not significant). Serum levels of AECAs appeared less suppressible by cyclophosphamide therapy compared with ANCAs, and patients who were persistently positive for AECAs despite being ANCA-negative during remissions were at risk of subsequent relapse. Disease recrudescense was not observed in patients persistently tested negative for both AECAs and ANCAs. Intravenous immunoglobulin therapy was used in four patients and resulted in clinical improvement in all cases, but with variable changes in the levels of AECAs and ANCAs. We conclude that AECAs are commonly detected in patients with systemic vasculitis and their levels show a relationship to disease activity similar to that for ANCAs.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

[Adamantiades-Behçet disease: necrotizing systemic vasculitis with a fatal outcome].

Vascular involvement in Behçet's disease was first mentioned by Adamantiades and occurs in addition to the classic symptom triad. In the present work an unusually fulminant course of Adamantiades-Behçet disease with a lethal outcome is reported in a 43-year-old male patient of German origin. The first symptoms were painful oedemas of the joints, followed 1 year later by bipolar aphthous lesions and by multiple progressive ulcers of the skin and the oral mucosa. Histologically, pustule formation with underlying vasculitis was diagnosed. In spite of systemic treatment with azathioprine and high-dosed prednisolone, the course was progressive with multiple necrotizing ulcers, and the patient died 18 months after the onset of the disease with signs of heart insufficiency and cachexia. The autopsy findings revealed multiple lesions of ulcerating systemic vasculitis of the skin and other visceral organs, including the lungs and the central nervous system, and also endocarditis verrucosa ulcerosa of the mitral valve.

Adult↗
PubMed1990

Enhanced expression of the tumour necrosis factor/cachectin gene in peripheral blood mononuclear cells from patients with systemic vasculitis.

The expression of tumour necrosis factor-alpha (TNF-alpha) gene in peripheral blood mononuclear cells from patients with systemic vasculitis, periarteritis nodosa and Wegener's granulomatosis, has been studied by RNA dot blot and Northern blot assays. We further examined the transcriptional level of TNF-alpha gene in peripheral blood mononuclear cells from these patients by performing nuclear run on transcription assay. We demonstrate enhanced TNF-alpha gene expression in mononuclear cells from these patients compared with healthy subjects and patients with bronchial asthma. These findings suggest that the enhanced transcription of TNF-alpha gene in peripheral blood mononuclear cells from patients with systemic vasculitis may be involved in the pathophysiology/pathogenesis of these diseases by cytokine dysregulation.

Adult↗
PubMed2004

Isolated PACNS-like presentation of a systemic vasculitis complicating a myelodysplastic syndrome.

Myelodysplastic syndromes (MDS) are a series of haematological malignancies ranging from chronic refractory anaemia to leukaemia. There is increasing recognition of immunological abnormalities in patients with MDS, including few reports of cutaneous vasculitis; in no instance, a cerebral localization has been ascertained. Here, the case of a patient with MDS who presented exclusively with neurological signs that were considered indicative of a primary, isolated central nervous system vasculitis (PACNS) is reported. Although histological findings on brain tissue confirmed a small-vessel vasculitis, this had to be considered in the context of a systemic vasculitis. In fact, at autopsy, an involvement of skin, myocardium, lungs, liver, kidney and bone marrow was also found. An autoimmune vasculitis should be included in the differential diagnosis of acute-onset, isolated, cerebral symptoms complicating the course of MDS.

Aged↗
PubMedDate not supplied

Catastrophic visceral involvement secondary to de novo systemic vasculitis in a renal transplant recipient.

De novo systemic vasculitis after renal transplant is a rare complication. We report a patient who developed rapid, catastrophic necrotizing vasculitis of the gastrointestinal tract 11 months after renal transplant. A 60-year-old man was admitted for persistent pain in the right abdomen and mild intestinal hemorrhage. After some days the patient presented partial intestinal occlusion, severe hypoproteinemia and acute renal insufficiency. The patient was urgently operated with resection of a tract of the jejunum where there was a venous infarct. Laboratory tests were not significant and the search for hepatitis B, C viruses and ANCA was negative. After some days of irrepressible intestinal hemorrhage, total gastrectomy, splenectomy and resection of the duodenum and pancreas were performed. Histological pictures showed vascular lesions pathognomonic of systemic polyarteritis nodosa (PAN). After thirty days the patient died. The autopsy confirmed atypical systemic PAN with involvement of the pulmonary arteries besides the gastrointestinal tract and pancreas. Gastrointestinal (GI) complications are not frequent in renal transplant recipients, but 30% of patients with such lesions die as a direct consequence of visceral vascular damage. To the best of our knowledge, de novo PAN has been reported as the cause of catastrophic gastrointestinal involvement in only one renal transplant recipient. This case therefore raises the number of reports of PAN in kidney transplantation recipients who had no history of underlying connective tissue disease.

Fatal Outcome↗
PubMed2002

Acute posterior multifocal placoid pigment epitheliopathy and scleritis in a patient with pANCA-positive systemic vasculitis.

PURPOSE: To present acute posterior multifocal placoid pigment epitheliopathy and scleritis occurring in association with pANCA (perinuclear antineutrophil cytoplasmic antibody)-positive systemic vasculitis. DESIGN: Interventional case report. METHODS: A 69-year-old woman developed bilateral acute posterior multifocal placoid pigment epitheliopathy concurrent with rapidly progressive glomerulonephritis and bronchioalveolitis in both lungs. RESULTS: The patient showed a high titer of pANCA (myeloperoxidase-ANCA). She then developed scleritis in the left eye and multiple gastric ulcers. CONCLUSION: Acute posterior multifocal placoid pigment epitheliopathy and scleritis in this patient are manifestations that are part of pANCA-positive systemic vasculitis. This association further supports that acute posterior multifocal placoid pigment epitheliopathy is caused by vasculitic inflammation of the choriocapillaris.

Acute Disease↗
PubMed1997

alpha 1-Antitrypsin (AAT) deficiency and ANCA-positive systemic vasculitis: genetic and clinical implications.

A high incidence of alpha 1-antitrypsin (AAT) deficiency has been reported in patients with C-ANCA systemic vasculitis in association with antibodies against proteinase-3 (PR3). To clarify the role of AAT deficiency in the acute vasculitic process as well as in progression of the disease, we studied 84 patients with either C-ANCA or P-ANCA vasculitis with special reference to: (a) the AAT gene, (b) the phenotypic (Pi) variants and (c) the serum levels during both acute illness and remission. The PiZ gene was found in six patients (8% vs. 1.5% controls) irrespective of the type of autoantibodies (C-ANCA vs. P-ANCA). All PiZ patients displayed the ability to raise their AAT serum levels up to the normal range during acute illness. In contrast, 24 patients with the PiM phenotype presented low AAT serum levels during acute illness. In all these patients, the AAT levels returned to normal values during the remission. Low AAT levels were associated with low levels of C-reactive protein (PCR) (P < 0.001), with a less severe renal involvement or a minor risk of death, and, in one tested patient, with a novel point mutation (TCGA-->TCAA) at the enhancer-promoter region of the AAT gene. Low AAT serum levels did not correlate with either type/titre of autoantibody or distribution/severity of the vasculitis process. In the case-control study, high AAT levels emerged as a major determinant of progression towards end-stage renal failure [odds ratio 3 (95% CI 1.1-8.4)]. These results indicate: (a) a high incidence of the PiZ gene of AAT in systemic vasculitis irrespective of the type of autoantibodies; (b) a novel form of AAT deficiency associated with the normal PiM phenotype becoming manifest only during acute illness; (c) dysregulation of the acute-phase response affecting selectively AAT or both AAT and PCR; (d) correlation between low plasma levels of AAT and less severe renal involvement or risk of death.

Adult↗
PubMed2006

Systemic vasculitis revealing a benign tumor: a paraneoplastic syndrome?

Systemic vasculitis is a rare event in the course of malignant tumors and has not been described in association with benign tumors. We report a case of renal oncocytoma in a patient who presented with digital necrosis, arthralgia, myalgia, and a decline in general health. The symptoms resolved fully after tumor excision, supporting a diagnosis of paraneoplastic syndrome.

Adenoma, Oxyphilic↗
PubMed2005

Disseminated epitheloid hemangioendothelioma mimicking symptoms of systemic vasculitis.

BACKGROUND: Epitheloid hemangioendothelioma is a rare malignant tumor which can involve bones, liver, lungs, kidneys, deep soft tissue, muscles, dermis, and central nervous system. Multifocal disease occurs in 10% of the cases. The clinical presentation results from occlusion of small blood vessels due to the disease itself or as a paraneoplastic syndrome. CASE REPORT: We present a patient with symptoms suggesting systemic vasculitis (ESR and CRP elevated, weight loss, arthralgia, livedoid rash, and skin ulcerations) who finally was diagnosed having a disseminated epitheloid hemangioendothelioma when PET scan revealed hypermetabolic multifocal skeletal and soft tissue lesions. DISCUSSION: Diseases mimicking systemic vasculitis and the value of PET in this setting are discussed.

Aged↗